Alien Hand Syndrome

Hassan, Anhar; Josephs, Keith A.

doi:10.1007/s11910-016-0676-z

Cited by 69 publications

(70 citation statements)

References 83 publications

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“…1, 32 The corticobasal syndrome is characterized by the presence of asymmetric clinical features that suggest a combination of cortical and subcortical (basal ganglia) pathology. Cortical dysfunction can manifest as the alien limb phenomenon 33 (in which the patient has lost control over a limb) attributed to involvement of sensory motor cortices and connections. Patients may personify their limb and sometimes will refer to their limb as “my little friend”.…”

Section: Clinical Diagnosis Of Diseases Considered Primary Tauopathiesmentioning

confidence: 99%

Current Understanding of Neurodegenerative Diseases Associated With the Protein Tau

Josephs

2017

Mayo Clinic Proceedings

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The primary tauopathies are a group of neurodegenerative diseases in which tau is believed to be the major contributing factor of the neurodegenerative process. In the primary tauopathies, there is a disassociation between tau, a microtubule associated protein, and microtubules, as a result of tau hyperphosphorylation. This disassociation between tau and microtubules results in tau fibrillization and inclusion formation, and microtubule dysfunction. There are different clinical syndromes associated with different primary tauopathies, and some clinical syndromes can be associated with multiple primary tauopathies. Hence, while some clinical syndromes are highly specific and almost diagnostic of a primary tauopathy, many are not, making it difficult to diagnose a primary tauopathy. Recently, radioligands that bind to tau and can be combined with positron emission tomography to detect fibrillary tau antemortem have been developed, although preliminary data suggest that these ligands may not be very sensitive in detecting tau associated with many of the primary tauopathies. Another recent advancement in the field is evidence demonstrating that tau may show similar properties to prions although infective transmission has not been demonstrated. There have been a few clinical trials targeting tau and microtubule dysfunction, although none have had any disease modifying effects. Understanding tau biology is critical to the development of pharmacologic agents that could have disease modifying effects on the primary tauopathies.

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Section: Clinical Diagnosis Of Diseases Considered Primary Tauopathiesmentioning

confidence: 99%

Current Understanding of Neurodegenerative Diseases Associated With the Protein Tau

Josephs

2017

Mayo Clinic Proceedings

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“…Cognitive impairment during the course of the disease occurs in 70% of cases and 52% had this symptom at presentation 25 . Cognitive dysfunctions in CBD will be discussed in detail, further in this review.…”

Section: The Clinical Spectrum Associated With Corticobasal Degenerationmentioning

confidence: 99%

Cognitive dysfunction in corticobasal degeneration

Oliveira

Barcellos

Teive

et al. 2017

Arq. Neuro-Psiquiatr.

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Corticobasal degeneration (CBD) is a rare and progressive neurodegenerative disease. It was originally described as a distinct clinicopathological entity in 1967 1 and later recognized as a polymorphous disease due to its complexity. In fact, the classical set of symptoms -namely asymmetric motor features associated with higher cortical function -were found to be associated with multiple histological presentations. Furthermore, the distinctive histopathological findings of cortical and striatal neuronal deposition of tau, and glial inclusions, were also linked to a wide variety of clinical phenotypes 2 . Hence, recent literature refers to corticobasal syndrome (CBS) as the constellation of symptoms described originally, limiting CBD to the distinctive histopathological findings .The different terminology with a seemingly paradoxical intrinsic overlap between these conditions makes diagnosis challenging. For instance, only 25% to 56.25% of patients with pathologically-confirmed CBD had previously presented with symptoms of CBS 4,5,6 . On the other hand, the proportion of patients diagnosed with CBS presenting with the histologic hallmarks of CBD is highly variable, ranging from 23.8% to 100% 4,5,6,7 . Considering the enormous difficulty in accurately diagnosing CBD during life, it is clear that current ABSTRACTCorticobasal degeneration (CBD) was originally described as a distinct clinicopathological entity in 1967. Since then, different phenotypic presentations have emerged as possible manifestations of CBD histopathological findings. In addition, pathophysiological findings and the molecular basis have been delineated and several aspects of its cognitive manifestations have been clarified. Thus, not only the spectrum of what is currently designated as CBD has expanded, but overlap with other degenerative and even secondary disorders has made clinical diagnostic certainty even more challenging in the absence of specific and readily-available markers. Cognitive deficits in CBD are now recognized as a frequent initial presentation and may appear up to eight years before the motor symptoms, depending on the phenotypic variant. Characteristic cognitive features of CBD involve language deficits, visuospatial and executive dysfunctions, apraxia, and behavioral disorders. Semantic and episodic memories are usually preserved, while language is often impaired in the early stages.Keywords: dementia; cognition; corticobasal degeneration. RESUMOA degeneração corticobasal (DCB) foi originalmente descrita como uma entidade clínico-patológica distinta em 1967. Desde então, nossa compreensão sobre DCB evoluiu substancialmente. Diferentes apresentações fenotípicas emergiram refletindo possíveis manifestações das anormalidades histopatológicos da DCB. Adicionalmente, dados fisiopatológicos e moleculares foram delineados e aspectos das manifestações cognitivas foram explorados. Assim, não só o espectro do que é atualmente designado DCB foi expandido, mas a sobreposição com outras doenças degenerativas e até mesmo secundárias to...

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“…The alien limb syndrome (ALS) was first described in 1908 by Goldstein [1], who defined ALS as a neurological condition characterized by "a stranger and extraneous feeling, typically about one arm, less frequently about one leg, and objective uncontrolled and unintentional movements." To date, three variants of the syndrome are known: the frontal, callosal and posterior variants [2]. The posterior syndrome is typically associated with the presence of unintentional and uncontrolled levitation of the affected limb whereas the frontal and callosal variants exhibit aberrant manipulation or intermanual conflicts [2].…”

Section: Introductionmentioning

confidence: 99%

Posterior Variant of Alien Limb Syndrome with Sudden Clinical Onset as Self-Hitting Associated with Thalamic Stroke

et al. 2020

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We present a case of sudden postischaemic onset of alien limb syndrome, with unintentional self-injury. Alien limb syndrome is an uncommon neurological disorder featured by uncontrolled and involuntary movements of a limb. Three variants of alien limb syndrome have been described: the anterior, featured by grasping of surrounding objects, the callosal, presenting with intermanual conflict, and the posterior, associated with involuntary levitation of the limb. Our patient suffered from an acute presentation of the posterior variant of the alien limb syndrome, resulting from an isolated thalamic stroke which was documented using 24-h Case

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Alien Hand Syndrome

Cited by 69 publications

References 83 publications

Current Understanding of Neurodegenerative Diseases Associated With the Protein Tau

Current Understanding of Neurodegenerative Diseases Associated With the Protein Tau

Cognitive dysfunction in corticobasal degeneration

Posterior Variant of Alien Limb Syndrome with Sudden Clinical Onset as Self-Hitting Associated with Thalamic Stroke

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